- Department of Neurosurgery, National Brain Aneurysm Center at the John Nasseff Neuroscience Institute, St. Paul, Minnesota, USA
- Minnesota Neurovascular and Skull Base Surgery, Minneapolis, Minnesota, USA
- Centra Care, St. Cloud Hospital, St. Cloud, Minnesota, USA
- Department of Pathology, Division of Neuropathology, Allina Health, Minneapolis, Minnesota, USA
- John Nasseff Neuroscience Institute, Director Stroke Care, Allina Health, United Hospital, St. Paul, Minnesota, USA
- HealthEast Care System, St. Paul, Minnesota, USA
Eric S. Nussbaum
HealthEast Care System, St. Paul, Minnesota, USA
DOI:10.4103/2152-7806.136702Copyright: © 2014 Nussbaum ES This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Nussbaum ES, Defillo A, Mcdonald W, Hanson S, Zelensky A. Diffuse idiopathic intracranial fusiform aneurysm development. Case report and literature review. Surg Neurol Int 11-Jul-2014;5:107
How to cite this URL: Nussbaum ES, Defillo A, Mcdonald W, Hanson S, Zelensky A. Diffuse idiopathic intracranial fusiform aneurysm development. Case report and literature review. Surg Neurol Int 11-Jul-2014;5:107. Available from: http://sni.wpengine.com/surgicalint_articles/diffuse-idiopathic-intracranial-fusiform-aneurysm-development-case-report-and-literature-review/
Background:Fusiform intracranial aneurysms (FIAs) are uncommon lesions representing less than 15% of all intracranial aneurysms in most large series. Their etiology has been linked to a variety of causes including atherosclerosis, fibromuscular dysplasia, cystic medial necrosis, connective tissue disease, hypertension, diabetes, hyperlipidemia, infection, cardiac myxoma, oral contraceptive use, vasculitis, and lymphoproliferative disorders. The finding of numerous lesions in a single patient is distinctly uncommon.
Case Description:We describe the unique case of a 47-year-old female who developed multiple FIAs over a 6-year period without an obvious underlying pathology. The patient's medical history was significant for obesity, migraine headaches, insomnia, breast cancer, and chronic skin rash. Various diagnoses were explored including infectious etiologies, autoimmune vasculopathies, malignancy-related causes, connective tissue disorders, and underlying genetic conditions. However, all investigations, including aneurysm wall and skin biopsies were negative or deemed noncontributory toward making a definitive diagnosis.
Conclusion:We report an unusual case of a patient with a normal cerebral angiogram developing numerous, FIAs without obvious underlying etiology over a 6-year period. Close clinical and radiological follow-up is recommended in this case because the natural history of the disease is unclear at this point. The literature regarding potential causes of multiple fusiform intracranial aneuryms is reviewed.
Keywords: Aneurysm, fusiform, idiopathic
Fusiform intracranial aneurysms (FIAs) are uncommon lesions representing less than 15% of all intracranial aneurysms in most large series.[
We describe the unique case of a 47-year-old female who developed multiple FIAs over a 6-year period without an obvious underlying pathology. The patient's medical history was significant for obesity, migraines, insomnia, breast cancer, and chronic skin rash. Various diagnoses were explored including: Infectious etiologies, autoimmune vasculopathy, malignancy-related possibilities, connective tissue disorders, and genetic diseases. However, all investigations, including aneurysm wall and skin biopsies were negative or deemed noncontributory toward making a definitive diagnosis.
A 47-year-old female developed confusion, headaches, and questionable seizure like activity. These symptoms prompted an emergency department visit at which time a computed tomography (CT) scan was performed and reported as unremarkable. She was left with a persistent dull headache and generalized weakness. Three weeks later, she developed a new episode of severe headache associated with photophobia, meningismus, nausea, vomiting, and dizziness. Her primary care physician ordered a magnetic resonance imaging (MRI), which showed scattered subarachnoid hemorrhage (SAH) located principally within the territory of the right middle cerebral artery (MCA).
The patient was promptly admitted to a hospital facility and underwent a computed tomography angiography (CTA), which demonstrated multiple elongated intracranial vascular abnormalities. These lesions involved both hemispheres including the anterior and posterior circulation. Of interest, 6 years prior to admission, she had presented with similar symptoms including the acute onset of severe headache. She had undergone a CT scan, which was reportedly negative, and a lumber puncture, which had demonstrated an elevated red blood cell count. This prompted a cerebral angiogram, which demonstrated normal intracranial vasculature without evidence of an aneurysm or other abnormality [
Past medical history was significant for migraine headaches, hypothyroidism, obesity (status postgastric bypass), and a diagnosis of breast cancer 4 years earlier with lumpectomy and radiation therapy. Family history was explored fully and was noncontributory in this case. In particular, there was no family history of aneurysm, stroke, connective tissue disorder, or other identified genetic issue condition within the family.
The patient was transferred to our facility and underwent catheter angiography, which revealed at least 20 fusiform aneurysms involving bilateral middle, anterior, and posterior cerebral arteries [
(a-e) Admission angiographic views of the left and right internal carotid arteries demonstrating multiple fusiform aneurysms involving the anterior and posterior divisions of both MCA M2-M3 segments.; (f-i) 3D reconstructions showing multiple fusiform aneurysms affecting all major intracranial arteries
The differential diagnosis for the dramatic development of multiple FIAs included hyper-IgE-related syndromes, autoimmune-inflammatory or malignancy-induced vasculopathy, connective tissue disease, genetic abnormalities affecting collagen production and structure as well as cardiac myxoma. The patient underwent numerous investigations including MRI [
The patient underwent microsurgical exploration through an extended right-sided pterional approach with wide splitting of the Sylvian fissure. The saccular component of the large right MCA branch aneurysm was confirmed as the source of bleeding and was clipped successfully [
(a) Hematoxylin and eosin. Original magnification ×100. Significant inflammation is absent. Well-developed distinction between intima, media and adventitia is not apparent. (b) Alcian blue (pH 2.5) Original magnification ×100. Alcian blue highlights acid mucosubstances and acetic mucins. Small amounts of staining are normal in blood vessel walls but increases in early lesions of atherosclerosis. The illustrated sample is essentially normal or mildly increased. (c) Verhoeff van Giesen elastic stain. Original magnification ×100. Note that no distinctive internal elastic lamina is visible within the sample
A literature search was performed using both PubMed and Medline search engines. The following word combinations were explored: “intracranial aneurysm”, “fusiform”, “diffuse”, and “idiopathic aneurysm formation”.
Multiple FIAs are exceedingly rare lesions. Possible etiologies suggested in previously reported cases have included Carney's syndrome, cardiac myxoma, viral infection (mostly due to Epstein-Barr and varicella-zoster), as well as a lymphocytic vasculitis reaction in x-linked lymphoproliferative syndrome.[
We have previously described a patient with numerous FIAs related to a cardiac myxoma.[
In the case of x-linked lymphoproliferative syndrome (Duncan's syndrome), the immune system is unable to properly combat infection by viral agents such as Epstein–Barr. The characteristic intracranial manifestation is a diffuse necrotizing vasculitis affecting the major arteries, primarily in the vertebrobasilar circulation.[
Because of a previous history of skin lesions in our patient, a possible diagnosis of autosomal dominant hyper IgE syndrome (AD-HIES) was entertained. This is a primary immune deficiency characterized by the classic triad of recurrent skin boils, cyst-forming pneumonias, and extreme elevations of serum IgE. Vascular abnormalities associated with this syndrome can include tortuosity and aneurysmal dilatation of mid-sized intracranial arteries, with SAH as an infrequent clinical sequela. Other recognized manifestations are eczema, mucocutaneous candidiasis, and several connective tissue and skeletal abnormalities. Both the actual disease and a variant disease-causing mutation were excluded in our patient. A variant of sex-linked lymphocytic necrotizing vasculitis was excluded as well.
We report an unusual case of numerous, diffuse FIAs without obvious underlying etiology. The fact that the patient had a normal cerebral arteriogram just 6 years earlier further adds to the unusual nature of the case. Close clinical and radiological follow-up is recommended in this case because the natural history of the disease is unclear at this point in time.
1. Ake JA, Erickson JC, Lowry KJ. Cerebral aneurysmal arteriopathy associated with HIV infection in an adult. Clin Infect Dis. 2006. 43: e46-50
2. Daugherty WP, Clarke MJ, Cloft HJ, Lanzino GL. Going viral: Fusiform vertebrobasilar and internal carotid aneurysms with varicella angiitis and common variable immunodeficiency. J Neurosurg Pediatr. 2009. 4: 528-31
3. Dutz JP, Benoit L, Wang X, Demetrick DJ, Junker A, de SA D. Lymphocytic vasculitis in x-linked lymphoproliferative disease. Blood. 2001. 97: 95-100
4. Jaworska K, Dolowy J, Kusmierska M, Kuniej T, Jazwiec P. Multiple fusiform cerebral aneurysms – case report. Pol J Radiol. 2012. 77: 50-3
5. Jean WC, Walski-Easton S, Nussbaum ES. Multiple intracranial aneurysms as a delayed complication of atrial myxoma: Case report. Neurosurgery. 2001. 49: 200-3
6. Loeffel S, Chang CH, Heyn R, Harada S, Lipscomb H, Sinangil F. Necrotizing lymphoid vasculitis in x-linked lymphoproliferative syndrome. Arch Pathol Lab Med. 1985. 109: 546-50
7. Maeda E, Akahane M, Kiryu S, Kato N, Yoshikawa T, Hayashi N. Spectrum of Epstein-Barr virus-related diseases: A pictorial review. Jpn J Radiol. 2009. 27: 4-19
8. Murakami K, Ohsawa M, Hu SX, Kanno H, Aozasa K, Nose M. Large vessel arteritis associated with chronic active Epstein-Barr virus infection. Arthritis Rheum. 1998. 41: 369-73
9. Ryou KS, Lee SH, Park SH, Park J, Hwang SK, Hamm IS. Multiple fusiform myxomatous cerebral aneurysms in a patient with Carney complex. J Neurosurg. 2008. 109: 318-20
10. Santillan A, Sigounas D, Fink ME, Gobin YP. Multiple fusiform intracranial aneurysms 14 years after atrial myxoma resection. Arch Neurol. 2012. 69: 1204-5
11. Sedat J, Chau Y, Dunac A, Gomez N, Suissa L, Mahagne MH. Multiple cerebral aneurysms caused by cardiac myxoma. A case report and present state of knowledge. Interv Neuroradiol. 2007. 13: 179-84
12. Vates GE, Auguste KI, Lawton MT, Le Roux PD, Winn HR, Newell DW.editors. Fusiform, dolichoectatic and dissecting aneurysms: Diagnosis and management. Management of Cerebral Aneurysms. USA: Elsevier Saunders; 2004. p. 689-709
13. Weeks JK, Helton KJ, Conley ME, Onciu M, Khan RB. Diffuse CNS vasculopathy with chronic Epstein-Barr virus infection in x-linked lymphoproliferative disease. AJNR Am J Neuroradiol. 2006. 27: 884-6